17alpha-OH-Progesterone (17alpha-OH-P) is a steroid produced in the adrenal gland and gonads and has no defined physiological role except as a precursor molecule. In the adrenal glands, 17alpha-OH-P can be converted to cortisol by the sequential actions of 21-hyroxylase and 11-hyroxylase. In both the adrenal glands and ovaries, 17alpha-OH-P can be converted to androstenedione (a precursor for testosterone and estradiol), by 17,20-lyase. Measurement of circulating 17alpha-OH-P levels is a standard tool for clinical assessment of 21-hydroxylase deficiency, the most common cause of congenital adrenal hyperplasia (CAH) [1,2,3]. Due to the decreased activity of 21-hydroxylase in CAH, 17alpha-OH-P cannot be efficiently converted to cortisol and instead, accumulates in large amounts which are shunted into the androgen biosynthetic pathway. The resulting elevations in androgen levels cause progressively severe virilization beginning during fetal life and infancy. In addition, 21-hydroxylase is required for aldosterone synthesis, and approximately 50% of cases of 21-hydroxylase deficiency suffer from potentially fatal salt-wasting. Elevated serum 17alpha-OH-P levels are found in virtually all cases of classical 21-hydroxylase deficiency. 17alpha-OH-P levels are useful in monitoring steroid replacement therapy [1,2,3].

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